1-.

A 5-month-old boy presents with florid red rash over his scalp, nappy area and
trunk.
Despite this he seems well in himself and feeding well. He was born at term weighing
3.1 kg and there were no neonatal problems. He is fully immunised and there is no
FH/SH of note.
On examination he is apyrexial and well. The rash is florid red and confluent over his
nappy area. He has a crusty confluent covering over his scalp, extending onto his
forehead. Smaller 0.5-1 cm greasy lesions are present over his trunk.
What is the most likely diagnosis?
(Please select 1 option)
Acrodermatitis enteropathica
Ammoniacal dermatitis
Histiocytosis
Infantile eczema
Seborrhoeic dermatitis Correct
The picture is of extensive greasy scaly rash especially over the head (cradle cap) and
nappy area without systemic upset.
This is highly characteristic of seborrhoeic dermatitis.
Selenium shampoo and topical steroids usually result in rapid resolution.

2- A 3-year-old girl presents with daily high fever, rash and joint pain of six weeks
duration.
The rash appears at the height of fever. The knees, ankles and wrists are affected.
She had a full term normal delivery with no neonatal problems. Her immunisations
are up to date. There is no family or social history of note.
On examination the temperature is 38.3C, and she has a florid erythematous rash
over the trunk, face and limbs. There is generalised lymphadenopathy, with 3 cm liver
and 3 cm spleen palpable. The affected joints are warm, swollen, tender and limited in
movement.
What is the most likely diagnosis?
(Please select 1 option)

Henoch-Schnlein purpura
Kawasaki disease
Rheumatic fever
Septic arthritis
Systemic juvenile arthritis Correct
The duration and degree of joint inflammation suggest a chronic arthritis.
The systemic symptoms of fever and rash make the most likely diagnosis systemic
juvenile idiopathic arthritis (Still's disease).

3- An 18-year-old woman presents with red, tender lumps on her shins and arthralgia.
Chest x ray shows bilateral hilar lymphadenopathy and clear lung fields. A clinical
diagnosis of sarcoidosis is made.
Which one of the following is the most appropriate management plan?
(Please select 1 option)
24 hour urinary calcium measurement Incorrect answer selected
Follow up appointment with chest x ray in three months This is the correct
answer
Mediastinoscopy and lymph node biopsy
Skin biopsy
Thoracic CT scan
This is also known as Lfgren's syndrome (a benign form of sarcoidosis).
The presentation of erythema nodosum, arthropathy, bilateral hilar lymphadenopathy
syndrome is so characteristic that histological diagnosis is not necessary.
The prognosis is excellent with less than 10% having persistent disease.
With less characteristic presentations, positive biopsies are needed.
Mediastinoscopy is the method of choice for anterior mediastinal nodes.

4-A 12-year-old boy presents with a florid rash on the hands and feet. He became ill
three days before, when he developed painful mouth ulcers. Yesterday he developed a
rash on the hands and feet. He was a full term normal delivery, and previously has
been very healthy. He is on no medications, is fully immunised, and there is no family
history of note.
On examination he has a temperature of 38.5C, respiratory rate 15/min and heart rate
is 85/min. He has profuse oral ulcers on his tongue and buccal mucous membranes.
He has many circular raised pinky red lesions over the hands and feet with darker
centres. He has tender cervical lymphadenopathy.
What is the most likely diagnosis?
(Please select 1 option)
Erythema multiforme This is the correct answer
Hand, foot and mouth disease
Infectious mononucleosis Incorrect answer selected
Kawasaki disease
Staphylococcal scalded skin syndrome
The history suggests an attack of oral ulceration, followed by the development of
target lesions over the extremities.
The likely diagnosis is primary herpes 1 infection, complicated by erythema
multiforme.
Treatment is supportive, though acyclovir may be given to control the herpes.

5- A 10-month-old girl is referred with a florid skin rash.

Soon after birth she developed cradle cap and dry skin on the cheeks. This gradually
improved, but over the past few months she has had dry red skin over the trunk, the
elbows, wrists, knees, ankles and base of the ears. This is itchy and disturbs sleep.
She was born at 37+3/40 weighing 3.34 kg and there were no other neonatal
problems. She has been fully immunised. Mother has hay fever and asthma.
On examination she is on the 10th centile for height, weight and OFC. Temperature is
36.4C, RR 30/min and HR 100/min. She has a dry red scaly rash on patches of the
trunk, elbows, wrists, ankles and knees. She has enlarged glands in the axillae and
groins.
What is the most likely diagnosis?

(Please select 1 option)

Atopic dermatitis Correct
Contact dermatitis
Erythema multiforme
Impetigo
Stevens-Johnson syndrome
The type, distribution and progress of the rash, in conjunction with the family history,
suggest atopic eczema.
This is treated with moisturisation and topical anti-inflammatories (steroids), the
strength of the latter being titrated against effect.
Irritants and desiccants should be avoided.

6-. A 4-year-old boy presents with a perioral rash. This has been present for many
months, but is not resolving.
He was born at term and there were no neonatal problems. He has been well and
thriving on no medications. He is fully immunised to date and there is no FH/SH of
note.
On examination his temperature is 36.9C, HR 95/min, RR 20/min. He has dry flaky
skin around his mouth, which he licks frequently.
What is the most likely diagnosis?
(Please select 1 option)
Contact dermatitis Correct
Erythema multiforme
Impetigo
Stevens-Johnson syndrome
Viral exanthems
The history of local dryness associated with licking is a form of contact dermatitis.
Recurrent contact with irritant, such as saliva, bubble bath, detergents, soaps and
citrus juices inflame the skin. Sweat may result in a similar foot problem.
Treatment is with emollients and avoidance of the trigger.

7- A 7-month-old girl presents with fever and a rash.

She was completely well until five days ago, when she developed a slight cold. The
next day she developed fever to 39.7C, which has persisted despite antipyretics.
Despite this she has remained relatively well and continues to drink, though her
appetite is poor. Today she has developed a rash over the face and trunk.
She was born at term weighing 3.8 kg and there were no neonatal problems. She is
fully immunised to date and there is no FH/SH of note.
On examination she has a temperature of 36.8C, RR 25/min and HR 100/min. The
rash is macular, profuse, pink and blanching. It is most prominent over the face and
trunk. She has shotty cervical lymphadenopathy.
What is the most likely diagnosis?
(Please select 1 option)
Contact dermatitis
Erythema multiforme
Impetigo
Stevens-Johnson syndrome
Viral exanthems Correct
The history of a well child with high fever for a few days followed by resolution of
fever at around the time of appearance of a rose-coloured rash is characteristic of
roseola infantum.
Since the introduction of measles, mumps, rubella (MMR), this is by far the
commonest cause of a measles-like rash.
The peak incidence is six to eighteen months. 5% develop febrile seizures.
It is caused by human herpes virus 6 and 7.

8- A 2-week-old girl presents with a history of poor feeding, worse over the last six
hours. 38+3/40, 2.95 kg, no problems at birth. First pregnancy of healthy Caucasian
mother. No FH/SH of note. No drugs nor immunisations.
On examination temperature is 35.4C (tympanic), RR 60/min, HR 160/min on
monitor (all pulses impalpable). Cold mottled peripheries, capillary refill time six
seconds. Oxygen saturations 45% in air. Gallop rhythm, 4 cm hepatomegaly, no
spleen. No bruising or rash.
What is the most likely diagnosis?
(Please select 1 option)
Congenital adrenal hyperplasia
Escherichia coli septicaemia
Galactosaemia
Group B streptococcal infection
Hypoplastic left heart syndrome Correct
The history is of shock, hepatomegaly and hypoxia in a 2-week-old child.
The most likely diagnosis is duct-dependent congenital heart disease. Left heart
lesions causing this presentation include:

aortic coarctation/hypoplastic arch

truncus arteriosus
critical aortic stenosis
hypoplastic left heart syndrome.

Right-sided lesions include:

transposition
severe Fallot's
pulmonary atresia +/- VSD.

After intubation and ventilation the key step is to open the duct using prostaglandin
E2 infusion.
Acidosis may require separate correction.
Urgent transfer to a cardiac centre should then follow so a specific diagnosis can be
made.

9-A young child is noted to have a platelet count of 50 x109/L (150-400 x109). Which
one of the following condtions may explain the thrombocytopenia?
(Please select 1 option)
Disseminated intravascular coagulation Correct
Henoch-Schonlein purpura
Kawasaki disease
Leukaemia in remission
Vitamin K deficiency
The platelet count will be reduced in acute leukaemia due to bone marrow infiltration
by blasts, but is normal in remission.In Kawasaki disease there will be
thrombocytosis. Disseminated intravascular coagulation is characterised by low
platelets and abnormal clotting profile. In Henoch-Schonlein purpura there will be
normal clotting profile and platelet count. Vitamin K deficiency causes prolonged
Prothrombin time and no thrombocytopenia.
Next question

10-A 7-year-old boy presents with swelling under his arm. He was well until 7day
before, when he developed mild fever and malaise. Full term normal delivery, no
neonatal problems. Immunisations up to date. No family history of note. The family
have a cat.
On examination the temperature is 37.9C. He has a 3cm smooth enlargement of a
node in the right axilla. This is slightly red but not fluctuant. Otherwise there are no
abnormalities to find.
What is the most likely diagnosis?
(Please select 1 option)
Cat scratch disease Correct
Hodgkin lymphoma
Infectious mononucleosis
Kawasaki disease
Mycobacterial adenitis
The history suggests subacute regional gland enlargement due to inflammation. The
most likely diagnosis is cat-scratch disease, which is caused by Bartonella henselae.
The incubation period is 3-30 days and often small erythematous lesions can be found
along the scratch marks. Regional adenopathy develops after 1-4 weeks.
Next question

11-The following statements are true of pertussis:

True / False
It can occur in the first three months of life Correct
Absence of a whoop rules out the diagnosis in a child with a cough Correct
A lymphocytic leucocytosis should be demonstrated to confirm the diagnosis
Correct
Bronchiolitis obliterans is a recognised later complication Incorrect answer
selected
Ampicillin is the drug of choice to eradicate the infection Incorrect answer
selected
Pertussis can occur at any time in a child without immunity to the condition who is
exposed. The whoop may not be seen and apnoeic episodes can occur as a feature.
Although a lymphocytosis is observed the diagnosis is confirmed by culture of the
organism in nasal secretions. There is no reported association between bronchiolitis
obliterans. Erythromycin may assist in eliminating the organism from nasal
secretions.
Next question

12-Meningitis in the newborn baby:

True / False
May present with apnoeic attacks Correct
Is always associated with a bulging anterior fontanelle Incorrect answer
selected
Is most commonly caused by Haemophilus influenzae Correct
Is a risk factor for later conductive deafness Correct
Has an above average incidence in babies with a meningomyelocoele
Correct
Apnoeic episodes are a frequent presentation of sepsis in the newborn. The fontanelle
may be normal in the initial stages. The commonest aetiology in newborn babies is
group B streptococci, which may be acquired during or after delivery. The mortality is
5-15% in infants and of those who survive a proportion may be left with mental
retardation, speech problems, visual impairment and neural deafness rather than
conductive deafness. Meningomyelocoele is a risk factor for the introduction of
meningeal infection .
Next question

13-Idiopathic thrombocytopenia:
True / False
Is caused by hypoplasia of megakaryocytes in the bone marrow Correct
Results in a prolonged prothrombin time Correct
Is self-limiting over a 3-6 month period in the majority of affected children
Correct
Requires splenectomy in less than 20% of affected children Incorrect
answer selected
Is an indication for corticosteroid therapy whenever the platelet count falls
below 50 x 109/l Correct
The cause of idiopathic thrombocytopenia is not known but cases commonly follow a
viral infection. Prothrombin time is normal, but platelet function tests are abnormal.
The majority of cases will spontaneously remit. A minority of children go on to
require splenectomy. When required it is usually delayed till the child is older than 5
years to minimise childhood infections. The absolute platelet count is not a criteria for
immune suppression, but if the child develops bleeding complications steroids are
usually started.
Next question

14-Characteristics of thalassaemia major include:

True / False
Polycythaemia Correct
Low levels of foetal haemoglobin Correct
Splenomegaly Correct
High serum iron level Correct
Hair on end appearance on x-ray of the skull Correct
Anaemia would result rather than polycythaemia. HbF and HbA2 levels will be high.
Hepatosplenomegaly occurs secondary to chronic haemolysis and iron overload
(multiple transfusions) occur leading to the risk of cirrhosis. Ineffective erythropoesis
leads to excessive abnormal bone growth with skull and maxillary deformities.
More detail on Thalassaemia...
Next question

15-An 11-year-old girl is brought into the accident and emergency department by the
paramedics, along with three similarly aged friends who made the call to the
emergency services. The girls friends have advised the paramedics that the girl had
disclosed to them that she had taken 24 paracetamol tablets the previous night and had
coughed up blood while they were playing at a local recreation ground.
You are in attendance to assess the child, who refuses to speak with you. She appeared
alert and talkative with her friends. She says that she does not wish for her parents to
be called, it was all a bad joke and she does not want any medical treatment.
What is the most appropriate action in this case?
(Please select 1 option)
Accept that this was a bad joke and discharge the child.
Agree with the child not to contact the childs parents but call social service to
report this case.
Assess whether the child is Gillick competent and discharge her only if she is
Gillick competent.
Speak with the child to assess her capacity and to advise her of the serious nature
of a paracetamol overdose and the witnessed blood, and the importance of
contacting her parents to attend. Correct
Try to establish a full history from the child, and perform necessary investigations
and treatment without the knowledge of the childs parents.
It would be unusual for an 11-year-old child to be considered to be Gillick competent
in such an situation, but even if the child were to be considered to be competent, her
refusal of treatment could be lawfully overridden by authority given by a person with
parental responsibility (such as a parent) case Re W (a minor) [1992] 4 All ER 627.
It is important that a practitioner act in the best interests of their patients at all times
and provide enough information to the patient, explaining the importance contacting
her parents.
In circumstances where the child was incompetent it would be necessary to provide
treatment in her best interests under the common law doctrine of necessity (This is
consistent with the common law principles of the doctrine of necessity set out in the
case of Re F [1990] 2 AC 1 and is consistent with ethical guidance published by the
GMC in its booklet Seeking patients consent: the ethical considerations (November
1998) at paragraph 18:
In an emergency, where consent cannot be obtained, you may provide medical
treatment to anyone who needs it, provided the treatment is limited to what is
immediately necessary to save life or avoid significant deterioration in the patients
health). Alternatively, were the child to be competent and refusing treatment
which was vital to protect life or serious harm, then urgent efforts should be made to
obtain authority for treatment from someone with parental responsibility or
occasionally through the courts; legal advice will almost certainly be required.
It may be considered appropriate to contact social services should there be a concern
that the child may be at risk of serious harm or abuse. It is important to review local

child protection guidance and be familiar with the DOH guidance What to do if you
are concerned a child is being abused (2003)

Question supplied by the Medical Defence Union.

16-Theme:Respiratory paediatrics
A Asthma
B Chronic lung disease of prematurity
C Congenital lobar emphysema
D Cystic Fibrosis
E Drug misuse
F Gastro-oesophageal reflux
G Hypogammaglobulinaemia
H Kartageners syndrome
I Primary ciliary dyskinesia
J Recurrent aspiration
Select the most likely diagnosis from the above list for the following cases:
A 6-year-old girl presents with productive cough, and is on the 3rd centile for height
and 0.4% for weight.

Correct

A 7-year-old boy has had a runny nose from birth. He presents to the ENT clinic with
recurrent sinusitis and cough. Chest X-ray is normal.

Correct

A 14-year-old boy who is a high performance athlete is on the 50th centile for height
and 9th centile for weight. He has a nocturnal cough and dyspnoea during training.

Correct
Cough and exercise induced symptoms are a common combination in many
respiratory disorders .A productive cough, especially associated with poor growth is
highly suspicious of Cystic Fibrosis. A runny nose from birth is unusual and indicative
of ciliary dysfunction, as are chronic otitis media and sinusitis. When associated with
dextraposition, this is known as Kartageners syndrome. Diagnosis is by examination
of nasal cilia .Asthma affects 10-15% of the paediatric population and presentation
can occur at any age. Dry cough, wheeze and obvious trigger factors along with a
history of atopy point towards the diagnosis. Competitive athletes need to inform
governing bodies of all treatments for asthma.

Next question

17-Theme:GCS in children
A 3
B 4
C 5
D 6
E 7
F 8
G9
H 10
I 11
J 12
What is the Glasgow coma Scale score for each of the following:
An 18 month old baby was involved in a car accident. He opens his eyes
spontaneously, is persistently irritable, and flexes to pain.

Incorrect - The correct answer is 10

Eyes 4, verbal 3, motor 3
A 12-year-old boy fell off a scrambler bike. He opens his eyes to pain, speaks
incomprehensibly, and withdraws to pain.

Correct

Eyes 2, verbal 2, motor 4

A 7-year-old girl falls off a swing. She is opens her eyes to voice, is confused, and
localises pain.

Incorrect - The correct answer is 12

Eyes 3, verbal 4, motor 5
Glasgow Coma Scale (4-15 years)
EYE OPENING spontaneously 4
To verbal stimuli 3
To pain 2
No response 1
BEST MOTOR RESPONSE
Obeys verbal response 6
Localises to pain 5
Withdraws from pain 4
Abnormal flexion (decorticate) 3
Abnormal extension (decerebrate) 2
No response 1
BEST VERBAL RESPONSE
Orientated and converses 5
Disorientated and converses 4
Inappropriate words 3
Incomprehensible sounds 2
No response 1

Childs Glasgow Coma Scale (<4yrs)

EYE OPENING
Spontaneous 4
To verbal stimuli 3
To pain 2
No response 1
BEST MOTOR RESPONSE
Spontaneous/obeys verbal commands 6
Localises to pain or withdraws from touch5
Withdraws from pain 4

Abnormal flexion to pain 3

Abnormal extension to pain 2
No response 1
BEST VERBAL RESPONSE
Alert, babbles, coos as usual 5
Less than usual words, spontaneous irritable cry 4
Cries only to pain 3
Moans to pain 2
No response 1
The GCS is always the best possible response. The younger children are difficult, and
the modified childs score is used.
Next question

18-Theme:Language disorders.
A Acquired hearing defect
B Autism
C Asperger syndrome
D Congenital deafness
E Expressive language disorder
F Landau-Kleffner syndrome
G Pervasive developmental disorder
H Selective mutism
I Specific articulation disorder
For each scenario choose the most likely diagnosis:
A 2-year-old boy is referred with poor communication. He avoids eye contact and
plays obsessively with a small range of toys. He does not speak.

Correct
The 2-year-old boy has autism, with defective language, poor reciprocal
communication and obsessional behaviour.
A 5-year-old girl is referred with loss of speech. She previously spoke sentences, but
now has difficulty both understanding and speaking.

Incorrect - The correct answer is Landau-Kleffner syndrome

The 5-year-old girl has lost speech. This may be associated with fits, and is
characteristic of the Landau-Kleffner syndrome.
A 6-year-old girl refuses to speak in class. She converses normally at home.

Correct
This 6-year-old girl has context-dependent speech problems, so-called which is an
anxiety disorder.

19-Concerning leukotrienes, which of the following statements is/are true/false?

True / False
Act on intracellular receptors Correct
Are derived from palmitic acid Incorrect answer selected
Cause bronchodilation Correct
Generally increase vascular permeability Correct
Leukotriene C4 and leukotriene D4 are important components with slowreacting substance A Incorrect answer selected
Leukotrienes are produced, like prostaglandins, from arachidonic acid but via the
lipo-oxygenase pathway, while prostaglandins are produced via the cyclo-oxygenase
pathway.
Levels are increased in acute respiratory distress syndrome (ARDS).
Leukotriene E4 causes capillary leak in mevalonic aciduria. Leukotriene B4 activates
polymorphonuclear lymphocytes, and C4 is responsible for allergic sensitisation.
D4 inhibitors are useful in asthma. C4, D4 and E4 levels are increased in asthma
causing bronchus constriction and oedema.
Slow-reacting substance of anaphylaxis (SRSA) consists of leukotrienes C4, D4 and
E4.

Further reading:
Slow-reacting substance of anaphylaxis Ann R Coll Surg Engl. 1978 May; 60(3): 201204.
Copyright 2002 Dr Colin Melville
Next question

20-Spleen enlargement is invariably seen in:

True / False
Acute myeloid leukaemia Incorrect answer selected
Idiopathic thrombocytopaenic purpura Correct
Myeloproliferative disorders Incorrect answer selected
Polycythaemia rubra vera Correct
Subacute bacterial endocarditis Incorrect answer selected
A soft thin spleen may be palpable in 10% of neonates, 10% of normal children, and
5% of adolescents. In most individuals, the spleen must be 2-3 times its normal size
before it is palpable.
Common causes of splenomegaly include:

Infection:
o Bacterial: typhoid, endocarditis, septicaemia, abscess
o Viral: EBV, CMV and others
o Protozoal: malaria, toxoplasmosis
o Haematological: haemolytic anaemia (congenital or acquired),
extramedullary haematopoiesis, thalassaemia, osteopetrosis,
myelofibrosis.
Oncological:
o Malignant: leukaemia, lymphoma, metastatic disease
o Benign: haemangioma, hamartoma.
Infiltration/storage:
o Lipidoses: Niemann-Pick, Gaucher
o Mucopolysaccharidoses
o Infiltration: histiocytosis.
Congestion:
o Cirrhosis or hepatic fibrosis
o Hepatic, portal or splenic vein obstruction
o Congestive heart failure.
Cysts:

Congenital (true cysts)

acquired (pseudocysts).
Other: SLE, sarcoid, rheumatoid arthritis.
o
o

Although splenic enlargement is seen in all these conditions, this is not invariable.
However, in polycythaemia rubra vera, the diagnostic criteria are increased total red
blood cell volume, an arterial oxygen saturation of greater than or equal to 92%, and
splenomegaly. The disorder in this condition is that erythroid precursors do not
require erythropoietin to stimulate growth.
2002 Dr Colin Melville
Next question

21-Theme:Apnoea
A Blood cultures
B CSF culture
C CT scan head
D Drug screen on mother
E EEG
F Nasopharyngeal aspirate for viruses
G Nasopharyngeal aspirate for pertussis
H pH studies
I Urine metabolic screen
For each scenario choose the most specific diagnostic investigation:

A 5 day old girl presents to casualty following a brief apnoeic episode. On

examination she is apyrexial, sweating, has a high-pitched cry and is difficult to settle.

Incorrect - The correct answer is Drug screen on mother

The 5day old presents with apnoea in the absence of fever or structural problem. The
other symptoms point towards drug withdrawal, so a drug screen will be diagnostic.
A 4 month old girl presents with a 12 hour history of poor feeding and fever. She is
rushed to hospital after she stops breathing. On examination she has an Oxygen
saturation of 94%, and responds to pain. Serum glucose is 3.4 mmol/l.

Incorrect - The correct answer is Blood cultures

The 4 month old girl presents with a brief history of fever followed by apnoea. This
suggests an infective cause. The decreased level of consciousness mean that LP
should not be done yet. Blood cultures are likely to be the most helpful test.
A 5 month old boy has a 2 day history of coryza and poor feeding. On the day of
admission he has a brief period of apnoea and is rushed to hospital. On examination
he has 2 cm hepatomegaly and a blood glucose of 0.8 mmol/l.

Incorrect - The correct answer is Urine metabolic screen

The 5 month old presents with apnoea associated with mild infection and
hypoglycaemia. This points to a metabolic cause, and a metabolic screen should be
most helpful.
Apnoea can be caused by 3 main groups of conditions: infections, obstructions, or
toxins/drugs.
Next question

22-Theme:Ear pain in children.

A Acquired cholesteatoma
B Acute mastoiditis
C Acute otitis media
D Dental problems
E Foreign body
F Furuncle
G Herpes zoster
H Temporo-mandibular joint disease
I Tonsillitis
For each scenario choose the most likely diagnosis:
A 3-year-old boy presents with fever and ear pain. He was treated last week with
antibiotics for acute otitis media, and initially improved. On examination he has
swelling and tenderness behind the ear.

Correct
The 3-year-old child had symptoms of acute otitis media, and now has mastoid
inflammation, mastoiditis.

A 14-year-old presents with a history of chronic ear pain and discharge. On

examination he has a right perforated eardrum with whitish protrusion through it.

Incorrect - The correct answer is Acquired cholesteatoma

In the 14-year-old, there are chronic middle ear problems and a visible cholesteatoma.
A 6-year-old girl presents with left ear pain. On examination there are vesicles in the
left ear canal and left facial weakness.

Correct
In the 6-year-old there are vesicles in the ear canal and facial weakness. This is likely
to be Herpes zoster (Ramsay-Hunt syndrome).
Ear pain can be associated with abnormal external ear findings, abnormal middle ear
findings or neither.
Next question

23-Theme:Bleeding/bruising.
A Bernard-Soulier syndrome
B Child abuse
C Chronic liver disease
D Disseminated intravascular coagulation
E Ehlers Danlos syndrome
F Glanzmann thrombasthenia
G Uraemia
H Vitamin K deficiency
I Von Willebrand disease
For each scenario choose the most likely diagnosis:
A 3 month old breast fed infant presents with fits and bruising. Haemoglobin 10.4
g/dl, White cell count 7.6x109/l, Platelets 298x109/l. Blood film Normal. Prothrombin
time ratio 2.3. Factor VIII and IX activity normal. Factors II, V, VII and X activity
reduced.

Incorrect - The correct answer is Vitamin K deficiency

In 2 month old infant the low levels of II,V, VII, IX suggest vitamin K deficiency,
with late-onset haemorrhagic disease most likely.
A 2-year-old girl presents with 12 hour history of lethargy, fever and bruising.
Haemoglobin 11.2 g/dl, White cell count 4.5x109/l, Platelets 43x109/l, prothrombin
time ratio 2.2, Activated partial thromboplastin time ratio 1.9. Thrombin Time 15s.
Fibrinogen 0.9 g/l

Correct
In the 2-year-old girl the generalised abnormalities suggest DIC in relation to severe
infection (eg meningococcal disease).
A 3-year-old boy presents with easy bruising and tissue-paper scars. Haemoglobin
12.1 g/dl White cell count 8.3x109/l, Platelets 275 x 109/L, with normal blood film.
Bleeding time is elevated with normal von Willibrand factor activity.

Incorrect - The correct answer is Ehlers Danlos syndrome

In 3-year-old boy, the clinical appearance plust elevation of bleeding time with normal
platelets and film suggest Ehlers Danlos. Without the tissue-paper scars Glanzmann
would have to be considered.
In the assessment of bleeding or bruising an FBC, blood film, and clotting screen will
make the diagnosis in most cases. Low platelets (thrombocytopaenia) are usually due
to infection, ITP or leukaemia. Prolonged bleeding time may be due to von
Willibrand's, platelet or connective tissue disorders. Raised APPT suggest specific
clotting disorder, raised PT liver or vitamin K problems, and raised TT hepatic or
renal disease. Generalised abnormalities are found in DIC and liver disease. If all tests
are normal then Child abuse or Ehlers Danlos should be considered.
Next question

24-Theme:Renal masses.
A Perinephric abscess
B Polycystic kidney disease
C Posterior urethral valves

D Prune-belly syndrome
E Renal cystic dysplasia
F Renal vein thrombosis
G Simple renal cyst
H Ureterocoele
I Wilms tumour
For each of the following case scenarios select the most likely diagnosis from the list
above:
On first day check a day old boy is noted to have mass centrally below the umbilicus
which is dull to percussion.

Correct
In the neonate, a palpable bladder should prompt an urgent scan to exclude posterior
urethral valves.
A 9 month old girl presents with a high fever. A tender mass is palpable in the right
flank. Urine dipstix is positive for blood and leukocytes.

Correct
In the 9 month old girl the symptoms and signs suggest an upper pole renal infection,
with perinephric abscess being most likely.
A 3 month old boy presents with abdominal mass in the left flank. Urine dipstix is
positive for blood.

Correct
In the 3 month old boy, haematuria and renal mass without fever suggest Wilms'
tumour. This may be associated with aniridia and hemihypertrophy.
Next question

25-In a 12-year-old adolescent girl with acute diabetic ketoacidosis:

True / False

Boluses of sodium bicarbonate are frequently necessary to correct metabolic

acidosis Correct
Nasogastric tube insertion should be considered Incorrect answer selected
A bolus of 4 units/kg of human actrapid should be given IV Correct
The estimated fluid deficit should be replaced over 24 hours Correct
Hypokalaemia frequently occurs in the acute phase Incorrect answer
selected
The management priorities in diabetic ketoacidosis are fluids, insulin, potassium,
acidosis, identification and treatment of any underlying cause.

Fluids: if there is shock, then boluses of 20 ml/kg of normal saline should be

given, followed by correction of the deficit over 48 hours. Over rapid
correction may result in cerebral oedema. Monitoring should involve Glasgow
Coma Scale, fluid inputs and outputs, regular checks for electrolytes,
creatinine acid base status, and CVP or urinary catheterisation if required. A
nasogastric tube is passed if there is vomiting or depressed consciousness to
reduce the gastric dilatation.
Insulin: no bolus is now normally given, and the insulin infusion is usually
started at 0.05-0.1 unit/kg/hour. The blood glucose should be monitored
regularly, with an aim to reduce the blood glucose by 2 mmol/hour. More
rapid reduction risks cerebral oedema.
Potassium: the initial potassium is usually high, but potassium will drop
following treatment with insulin, as potassium and glucose enter cells together.
Potassium replacement is therefore instituted as soon as urine pH is passed.
Acidosis: a degree of acidosis is inevitable, but bicarbonate should be avoided
unless the pH is less than 7 or is not improving. The acidosis will self-correct
with fluid and insulin therapy.
Other: infection may need antibiotics.

Copyright 2002 Dr Colin Melville

Next question

26-Burkitt's lymphoma:
True / False
Always presents with a mass in the jaw

Is highly responsive to chemotherapy

Correct

Correct

Is one of the commonest solid tumours of childhood in equatorial Africa

Correct

Occurs in endemic and non-endemic forms

Usually presents with stage III or IV disease

The Epstein-Barr virus is implicated in

Correct

Correct

Burkitt's lymphoma

lymphoepithelioma

Hodgkin disease.
90-95% of Burkitt's lymphoma in Africa are EBV related, versus only 20-30% in the USA.
Chronic stimulation of B lymphocytes by EBV can promote chromosomal translocations that contribute to malignant
transformation. Chronic malarial infection appears to increase the risk of Burkitt's lymphoma by decreasing immune
surveillance of genetically altered cells.
Burkitt's lymphoma is a small, non-cleaved cell (SNCC) type of non-Hodgkin's lymphoma (NHL). These are B cell
tumours that express cell surface immunoglobulin and contain one of three characteristic chromosomal
translocations: t(8;14), t(2;8), or t(8;22). Each involves the c-Myc oncogene and an immunoglobulin gene.
The endemic (African) Burkitt's lymphoma is often found in the jaw, and is the most common childhood cancer in
equatorial East Africa and New Guinea. The mean age of onset is five years.
Only 20% of non-endemic (sporadic) Burkitt's lymphoma cases contains EBV genomes. Prognosis is good, as for
most forms of non-Hodgkin's lymphoma (NHL).
Copyright 2002 Dr Colin Melville

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Tag Question

27-The following features are recognised in profound hypothermia:

True / False
Hypertension

Correct

Haemoglobinuria

Correct

Beneficial effect of Prednisolone

J wave on ECG

Correct

Correct

Ventricular arrhythmia

Correct

Hypothermia occurs particularly in winter sports injury, equipment failure, or exhaustion decrease the degree of
exercise. Immersion and wet wind chill rapidly produce hypothermia, defined as a rectal temperature less than 35C,
when the body can no longer sustain normal temperature through shivering. Clinical features include extreme
lethargy, fatigue, incoordination, apathy, which are followed by mental confusion, clumsiness, irritability,
hallucinations, and finally bradycardia. Ventricular arrhythmia can be a problem because of vibration during
transfers. J waves may be seen on the ECG. Haemoglobinuria may occur secondary to D.I.C.
Warming plus monitoring of blood pressure, fluid pH and oxygen status are necessary.
Copyright 2002 Dr Colin Melville

Next question

28-Regarding congenital hypothyroidism:

True / False
It is much less common than phenylketonuria.

Correct

It is due to dyshormogenesis in about 10% of cases.

selected

Incorrect answer

Hypothyroidism due to TSH deficiency is very rare.

Correct

Despite early treatment, a few patients have severe learning difficulties.

Correct

Neonatal screening detects >95% of cases.

Correct

The neonatal screening programme for congenital hypothyroidism is a major recent triumph for Paediatrics, with very
few cases missed. It is one of the most common of the metabolic defects, detection is virtually complete, and the
long term outcome should be an IQ within the normal range. Causes of congenital hypothyroidism include:

Athyrosis.

Maldescent.

Dyshormonogensis (10%).

Iodine deficiency (commonest worldwide, but rare in the UK because of iodination of salt).

TSH deficiency (usually associated with panhypopituitarism, very rare).

Copyright 2002 Dr Colin Melville

Next question

29-The following are features of congenital adrenal hypoplasia:

True / False
Low renin levels

Correct

Exaggerated diurnal cortisol responses

Correct

Association with Duchenne Muscular Dystrophy

Association with vitiligo

Incorrect answer selected

Incorrect answer selected

Presentation with diarrhoea, vomiting and dehydration

Correct

Hypoadrenalism usually presents in the neonatal period with increasing pigmentation, salt loss and cryptorchidism in
males. The disorder is due to a mutation in the DAX 1 gene, a new nuclear hormone receptor family, located on
Xp21.3-p21.2. Hypogonadotrophic hypogonadism (HHG) is caused by the same gene mutation, and the
cryptorchidism is explained by this. The disorder is located very close to the Duchenne muscular dystrophy gene and
the glycerol kinase gene, so these are associations.
Copyright 2002 Dr Colin Melville

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30-The following are true of angiotensin II:

True / False
It is 50 time more potent than angiotensin I as a renal arterial vasodilator.
Correct

It inhibits aldosterone secretion.

Correct

It reduces afterload in cardiac failure.

Correct

Angiotensin I is converted to angiotensin II in the kidney.

Correct

Secretion is increased by hypokalaemia.

Correct

Renin is produced by the juxtaglomerular apparatus of the kidney, and converts and alpha-2 globulin to angiotensin
I. In the lung angiotensin I is activated to angiotensin II, which is 50 times more potent as a presser agent than
noradrenaline. Angiotensin II acts directly on the adrenal cortex to stimulate secretion of aldosterone, a potent
mineralocorticoid produced in the zona glomerulosa. Renin secretion is increased by sodium deprivation, and
aldosterone acts at the distal tubule to control sodium reabsorption (and indirectly, water reabsorption).
Copyright 2002 Dr Colin Melville

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31-A 7-year-old child presents with a four month history of daily headache. This is frontal and is worsened by
coughing, sneezing and bending. He has a persistent purulent nasal discharge.
Full term normal delivery with no neonatal complications. Immunisations up to date. There is no FH/SH of note. On
examination he has a temperature of 37.3C, respiratory rate 15/min, pulse 90/min. Blood pressure is 80/60 mmHg.
He has a blocked nose with purulent discharge, and is tender over both maxillae. Ear and throat examination are
normal.
What is the most likely diagnosis?
(Please select 1 option)

Cerebral abscess
Chronic sinusitis

Correct

Cluster headache
Migraine
Tension headache
The history of persistent chronic headache with persistent nasal discharge and maxillary tenderness suggest sinusitis
affecting frontal and maxillary sinuses. This can be confirmed by CT scan. It is usually treated by antibiotics and
decongestants but surgical drainage may be required

32-The platelet count is typically reduced in:

True / False

Acute leukaemia
Kawasaki disease

Correct
Correct

Disseminated intravascular coagulation

Henoch-Schnlein purpura
Vitamin K deficiency

Correct

Correct

Correct

The platelet count will be reduced in acute leukaemia due to bone marrow infiltration by blasts.
In Kawasaki disease there will be thrombocytosis.
Disseminated intravascular coagulation is characterised by low platelets and abnormal clotting profile.
In Henoch-Schnlein purpura there will be normal clotting profile and platelet count.
Vitamin K deficiency causes prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT), but
not thrombocytopaenia.

The Prothrombin time is a measure derived from the Prothrombin Ratio (PR) and the International Normalised
Ratio (INR). This is used in checking if the dose of warfarin is correct. The prothrombin time measures the extrinsic
pathway of coagulation; this includes measuring Clotting Factors 2, 5, 7, 10 and Fibrinogen. The normal value is 1215 seconds (depending on your control).
The Activated Partial Thromboplastin time measures the intrinsic pathway of clotting. It measures the clotting
factors - 8, 9, 11, 12, and is used in Von Willebrand's disease. It is used to check if the dose of heparin is correct.
The normal value is 25-39 seconds (depending on your control).

33-A female infant is born at 28+4/40 gestation weighing 1.01kg. She requires 13day ventilation, 2day CPAP and
then continued nasal prong Oxygen at 0.02 l/min. At 8 weeks of age she still has this Oxygen requirement. Capillary
gases show pH 7.38, pCO2 9.5 kPa, base excess of +8 mmol/l. She is discharged home on Oxygen at 0.02 l/min.
4 weeks after discharge she presents to the Paediatric ward with respiratory distress. Her O2 saturation is 85% in
nasal prong Oxygen of 0.02 l/min. She has respiratory rate of 60/min, marked recession and heart rate of 165/min.
There is 2 cm hepatomegaly.
What is the most likely diagnosis?
(Please select 1 option)

Bronchiolitis

This is the correct answer

Bronchopulmonary dysplasia

Incorrect answer selected

Cardiac failure
Pneumonia
Wilson-Mikitty syndrome
She fulfils the definition of chronic lung disease, which is O2 dependence at 36/40 corrected age. This condition is caused by a
combination of O2 therapy, barotrauma (from ventilation) and fluid overload. The result is ongoing lung scarring, decreased
lung compliance and gas exchange. There is respiratory acidosis (CO2 retention) with compensatory metabolic alkalosis.
These infants have diminished lung reserve and are very vulnerable to respiratory infections, with a greatly increased risk of
PICU. Her second presentation is likely to represent an acute infection, most probably bronchiolitis.

34-A 4-year-old boy presents with dizziness and vomiting. He has been unwell over the past few days with fever,
right ear pain and discharge. Full term normal delivery with no neonatal complications. Immunisations up to date.
There is no FH/SH of note.
On examination he has a fever of 38.6C, RR 20/min and HR 100/min. Chest is clear. He has a purulent discharge
from his right ear. He has nystagmus, and walks with a broad-based gait.
What is the most likely diagnosis?
(Please select 1 option)

Brain tumour
Infections

Correct

Ingestions
The history suggests an acute otitis media with developing ataxia and vomiting. This suggests extension of the
infection into the vestibular apparatus. From there it can extend into the brain. IV antibiotics and surgical drainage
may be required.

35-1, 25, dihydroxycholecalciferol:

True / False

Causes suppression of parathyroid hormone (PTH) by direct action on the

parathyroid gland.

Incorrect answer selected

Causes suppression of parathyroid hormone indirectly by increasing serum

calcium.

Correct

Acts on cell surface receptors.

Correct

Is biologically more active than 25, dihydroxycholecalciferol.

Leads to increased osteoblast activity.

Correct

Incorrect answer selected

7, dihydrocholesterol is converted by sunlight into cholecalciferol (Vitamin D3). In the liver this is 25, hydroxylated,
and in the kidney this is 1, 25 hydroxylated. Vitamin D is not abundant in natural foods except for fish liver oils, fatty
fish and egg yolk. Ergocalciferol (Vitamin D2) is used to fortify margarine. 1, 25 dihydroxycholecalciferol is the most
active form of the vitamin. It is produced following parathormone secretion in response to a low plasma calcium. The
effect of 1, 25 (OH2D3) is to induce synthesis of a calcium binding protein (calbindin-D) in the intestinal mucosa with
the resultant absorption of calcium. It also promotes bone dissolution and mineralisation. Thyroid hormones also
mobilises calcium by directly enhancing bone resorption, an effect that requires 1, 25 dihydroxy Vitamin D. The
effects of PTH on bone and kidney are mediated through binding to specific receptors on the membranes of target
cells and through activation of the transduction pathway involving a G protein coupled with an adenylate cyclase
system. The active form of vitamin D binds to intracellular receptors that then function as transcription factors to
modulate gene expression.
Copyright 2002 Dr Colin Melville

36A mother is concerned because her one-year-old girl was in contact with measles 2 days previously. Which one
of the following statements is correct?
(Please select 1 option)

Nothing can be done to affect the course of the disease.

Gamma globulin can be administered up to 14 days after exposure.

Incorrect answer

selected
If she were to become infected then complications would be extremely rare.
Immunisation with the live attenuated virus will give long immunity if given
immediately

This is the correct answer

She should anticipate the rash occurring in the next 5 days.

If the person is within 72 hours of exposure measles vaccine is the treatment of choice as it will confer life long
immunity. In persons in whom the vaccine is contraindicated immunoglobulin can be given. Immunoglobulin is
effective within 6 days of exposure. One third of those afflicted with measles can expect a complication. These
include pneumonia, otitis media, diarrhoea. A serious complication is the development of sub-acute sclerosing, panencephalitis. The initial symptoms of measles are coryzal symptoms. Several days after the appearance of coryzal
symptoms a rash appears.

37-A 10-month-old Afro-Caribbean boy presents with painful swelling of the hands and feet. This has worsened
over the past six hours, making him distressed and weepy. Full term normal delivery born in Africa, no neonatal
problems, moved to the UK age 4 months. Immunisations up to date. Mother is known to have sickle trait.
On examination he is apyrexial, with respiratory rate of 30/min and pulse of 105/min. There is symmetrical swelling
of the hands and feet, which are painful to touch. His spleen is 3 cm below the costal margin.
What is the most likely diagnosis?
(Please select 1 option)

Haemophilia A
Kawasaki disease

Reactive arthritis
Septic arthritis
Sickle cell crisis

Correct

The history of symmetrical painful swelling of hands and feet in an Afro-Caribbean child suggests sickle cell dactylitis
('Hand and foot syndrome'), which is often the first manifestation of sickle cell disease. It is due to ischaemic
necrosis of the long bones as the bone marrow expands.
Haemoglobin electrophoresis confirms the diagnosis. Oxygen and analgesia should be given.

38-Theme:Stridor
A Acute Laryngitis
B Angioneurotic Oedema
C Bacterial Tracheitis
D Croup
E Diptheria
F Epiglottitis
G Foreign Body Inhalation
H

Retropharyngeal
Abscess

I Smoke Inhalation
J Vocal Cord Palsy
For each patient described below choose the single most likely option from the list above.
A 9 month old boy is referred to ENT as he has been noted to have stridor. He sometimes chokes when drinking or feeding. His OFC has
risen from the 50% at birth to the 98% and he has short limbs.

Correct

A 4-year-old girl attends a birthday party and develops a choking episode. She recovers, but over the next 2 days becomes more unwell
with a fever and a stridor. She is found to have marked intercostal recession.

Correct

A 3-year-old boy is brought to casualty with a sudden onset of stridor. Over the past 3 days he has been unwell with coryzal symptoms
and ear temperature of 38.2C.

Correct

The 9 month old child has achondroplasia, which is often associated with hydrocephalus (Arnold-Chiari
malformation). This in turn can be associated with vocal cord palsy.

Croup may be caused by different viruses although most commonly as a result of infection with Parainfluenza.
Typically it occurs in young children a few days after a coryzal illness with symptoms of a barking cough, a low grade
fever and an inspiratory stridor. Symptoms are worse at night.

The 4-year-old girl developing fever and stridor following a birthday party is a classical history for inhalation of a
foreign body e.g. a peanut. This results in deterioration over 24-36 hours and widespread respiratory signs.

39-Theme:Seizures
A Absence seizures.
B Atonic seizures.
C

Benign partial seizures with centrotemporal

spikes.

D Complex partial seizures.

E Febrile seizures.
F Generalised tonic clonic seizures.
G Infantile spasms.
H Myoclonic seizures.
I Simple partial seizures
J Status epilepticus.
For each of the cases below choose the single most likely option from the above list.
A 13-year-old girl complains of feeling unwell. She describes a strange taste in her mouth and suddenly becomes totally unresponsive.

Correct

A 9-year-old girl is seen to develop twitching over the left side of her face and neck. This lasts for approximately a minute and
throughout the episode is fully conscious.

Correct

A 9-year-old boy presents with episodes of facial twitching. His EEG shows a characteristic pattern in the temporal region.

Correct

The 9-year-old boy has benign partial epilepsy of childhood (BPEC). It is a common type of partial seizure with an
excellent prognosis. Age of onset is at 9-10 years with remission by 15 years. Seizures are partial with facial
twitching and drooling. EEG pattern is characteristic with centro-temporal spikes with normal background activity.
The 9-year-old girl has simple partial seizures (SPS) as she remains conscious throught the episode, whilst the 14year-old girl has complex partial seizures. In complex partial seizures (CPS) an aura is often experienced and
accompanied by automatisms. They may evolve into generalized tonic clonic seizures. In CPS there is a history of
impaired consciousness, unlike SPS where consciousness is maintained. In SPS asynchronous movements are seen
in the face, neck and extremities. An aura may be present but automatisms do not tend to exist.

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40-Intussusception in children:
True / False

Is more common between the age of 3 months to 3 years

Bile stained vomiting is an early feature

Correct

Correct

Bleeding per rectum is the most common presenting symptom

Incorrect

answer selected
Is usually caused by a polyp or pedunculated tumour

Incorrect answer

selected
Hydrostatic reduction plays an important role in the management

Correct

Intussusception is more common in the 3 month - 3 year age group. It occurs more commonly in males. Clinical
features include sudden paroxysmal colicky abdominal pain and vomiting. The vomiting is not bile stained. Bleeding
per rectum occasionally is seen but it is not common and is a late sign. Intussusception tends to occur in regions in
which a peristaltic segment abuts on to an aperistaltic segment. These include enlarged Peyers patches, Meckels
diverticula, tumours and haematomas complicating Henoch Schonlein purpura. In most cases the cause is not
identifiable

41-The following diagnoses can be reliably made on antenatal ultrasound performed before 20 weeks:
True / False

Spina bifida occulta

Gastroschisis

Incorrect answer selected

Correct

Ventricular septal defect

Gestational age
Down syndrome

Incorrect answer selected

Correct
Incorrect answer selected

Gestational age can be reliably estimated if performed before 20 weeks, but after this the margin for error increases.
Multiple pregnancies can be identified.
Up to 70% of major structural abnormalities can be identified, and more detailed scans and specialist centres
arranged.
Fetal growth can now be reliable measured from serial abdominal circumference, bi-parietal diameter and femur
length.
Oligohydraminos and polyhydraminos can also be diagnosed.

Although specialist centres can reliably diagnose major cardiac malformations, VSD can be very difficult to detect.
Nuchal fold thickness is being investigated as a possible means of making a diagnosis of Down syndrome, and results
are promising. However, the results are not currently reliable enough for routine screening.
Copyright 2002 Dr Colin Melville

42-Which of the following is true regarding sensory neural hearing loss?

True / False

The incidence is increased in babies admitted to neonatal intensive care units

compared with the normal population

Correct

The risk is increased in children who have had congenital rubella

Correct

Approximately 1 per 1000 children will have greater than 40 db hearing loss
Correct
The risk is increased in Noonan's syndrome
The risk is increased in Down syndrome

Incorrect answer selected

Incorrect answer selected

Sensory neural hearing loss is caused by lesions in the cochlea or the auditory nerve or central connections. It may
be unilateral or bilateral. Language acquisition and secondary educational difficulties follow, with social isolation, and
an increased risk of mental health problems. The approximate incidence is 1 per 1000 children. Risk factors include:

NICU admission: low birth weight, less than 32 weeks' gestation, prolonged ventilation,
prolonged jaundice, ototoxic drugs, hypoxic ischaemic encephalopathy, neonatal
meningitis.

Congenital infection (rubella, CMV).

Dysmorphic syndromes (affecting head and neck).

Family history of a close relative needing a hearing aid below the age of 5 years.

Infections: acute bacterial or TB meningitis, mumps (latter usually unilateral).

If all risk factors are considered, only around 50% of cases could be identified by testing between 5 and 10% of all
babies.
Conductive hearing loss is related to middle ear pathology. This is commoner in Down syndrome, cleft palate,
Turner's syndrome, and facial malformation syndromes. Down's children also have an increased risk of sensorineural
hearing loss.
In Noonan's syndrome the incidence of progressive high-frequency sensorineural hearing loss may be as high as
50%.
2002 Dr Colin Melville

43-Causes of lung cysts on chest x ray include which of the following?

True / False

Aspergillus infection

Correct

Congenital lobar emphysema

McLeod's syndrome

Correct

Correct

Staphylococcal pneumonia
Tuberculosis

Correct

Incorrect answer selected

Causes of lung cysts can be divided into:

Congenital:

lobar emphysema

cystic adenomatoid malformation

bronchogenic cyst

sequestration (intralobar, extralobar).

Acquired:

bronchiectasis

associations with any long-standing infection such as cystic fibrosis, post-measles, postpertussis, TB.

Staphylococcal and Klebsiella pneumonia are characteristically associated with cavitation.

Aspergillus usually, is happy to colonise cavities that are already present. Invasive pulmonary aspergillosis may
cause nodular infiltrates on chest x ray.
Copyright 2002 Dr Colin Melville

44-The following are characteristic presentations of cystic fibrosis:

True / False

A 26 week gestation infant with x ray appearance of meconium ileus

A 3-week-old term female with prolonged unconjugated jaundice

Correct

Incorrect

answer selected
An 8-month-old girl admitted the second time with lower respiratory tract
changes on chest x ray

Correct

A 9-month-old Indian girl with failure to thrive

Correct

A 4-year-old boy with mouth breathing due to nasal polyps

Typical clinical features at presentation are GI or respiratory.
Respiratory

Recurrent chest infections

Bronchiectasis

Pneumothorax

Sinusitis or nasal polyps

Correct

Haemoptysis

Aspergillosis.

Meconium ileus (neonate)

Steatorrhoea

Malabsorption

Failure to thrive or growth failure

Rectal prolapse

Prolonged conjugated jaundice.

Gastrointestinal

Copyright 2002 Dr Colin Melville

45-A black West African girl aged 15 years is complaining of severe pain in both legs. The haemoglobin is 8 g/dl.
If which of the following is true, is sickle cell anaemia unlikely to be the cause of her symptoms?
True / False

A Menarche occured at 12 years

B She has haematuria
C She is jaundiced

Correct

Correct

Correct

D The urinary osmolality is 800 mosm/kg (specific gravity approximately 1022)

Correct
E x Ray of the spine shows osteoporosis

Correct

A Growth and development are usually impaired.

B and C These are expected in sickle cell anaemia.
D Almost all are unable to produce concentrated urine.
E x Ray usually shows fish mouth vertebrae (infarcts).

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46-Do the following suggest pulmonary rather than aortic valve stenosis?
True / False

A deep S wave in V2

Correct

An ejection click best heard in the second and third left intercostal spaces
Correct
An ejection murmur radiating to the neck
An upright T wave in V1

Correct

Incorrect answer selected

Prominence in the left upper mediastinum on chest x ray

Correct

An ejection murmur radiating to the back (along the direction of the pulmonary arteries) is found in pulmonary
stenosis. The ejection click is found at the apex in aortic stenosis.
A deep S wave in V2 and large R wave in V6 suggests left ventricular hypertrophy (LVH), while an upright T wave in
V1 suggests right ventricular hypertrophy (RVH).
A prominent left upper mediastinum on chest x ray may be caused by post-stenotic aortic dilatation in aortic
stenosis.

47-The following features suggest tetralogy of Fallot rather than transposition of the great arteries:
True / False

Absence of clinical cyanosis

Absence of S wave in V1

Correct

Incorrect answer selected

Increased pulmonary vascular markings on chest x ray

Presentation at 2 months of age with a murmur
Single second heart sound

Correct

Correct

Correct

On chest x ray, a 'boot-shaped' heart with pulmonary artery bay and uptilted apex suggests Fallot's, while an 'egg on
side' appearance and pulmonary plethora suggests TGA.
In Fallot's lung blood flow is decreased, and the absence of an S wave in V1 suggests right ventricular hypertrophy.
The second heart sound is single in both conditions.
Transposition usually presents in the first few days of life, and the patient is invariably clinically cyanosed.
Copyright 2002 Colin Melville

48-Which of the following are true regarding a diagnosis of intussusception?

True / False

An abdominal mass

Incorrect answer selected

An empty rectum on examination

Fever

Incorrect answer selected

Correct

Fluid levels on an erect abdominal x ray

Increased bowel sounds

Correct

Correct

An infant presenting with sudden onset of colicky abdominal pain needs to be assessed for intussusception.
The baby may have a palpable abdominal lump in the right upper quadrant.
There is not usually a fever.

Abdominal x ray may show signs of small bowel obstruction.

The diagnosis is confirmed by ultrasonography, or with a barium enema which may reveal the claw sign at the point
of the bowel invagination.
Hydrostatic enema is the mainstay of non-operative treatment, whereby reduction can be achieved.

49-Which one of the following statements is true concerning airway obstruction in the newborn?
(Please select 1 option)

Epiglottis is a likely diagnosis

In Pierre Robin syndrome the airway can be improved by a nasopharyngeal tube
Correct
Stridor is less severe in the supine position
The commonest cause is subglottic stenosis
Vascular ring can be diagnosed by lateral x ray of the neck
The most common cause of neonatal stridor is laryngomalacia, characterised by low pitched inspiratory stridor. Other
causes include

unilateral vocal cord paralysis

subglottic stenosis.

In Pierre Robin syndrome there is

micrognathia

cleft palate

glossoptosis, where the tongue falls backwards and downwards.

Glossoptosis can be managed with a nasopharyngeal airway.

Epiglottitis is extremely uncommon now following the introduction of Haemophilus influenzae vaccine.
Stridor is more severe in the supine position and after agitation/crying.
Vascular ring is not diagnosed by x ray, but MRI can

50-Pneumocystis pneumoniae
True / False

Is an obligate intracellular organism

Incorrect answer selected

May be usefully diagnosed by serology

Correct

Can cause disease outside the respiratory system

Correct

Can have normal chest x ray with generalised wheeze

selected

Incorrect answer

Is adequately treated with erythromycin alone

Correct

Pneumocystis is an obligate extracellular parasite with attributes of both fungi and protozoa. Most humans are
seropositive by 4 years of age, though the transmission mode is unknown.
Forty percent of children with HIV and 10% with leukaemia get Pneumocystis carinii pneumonia (PCP) if no
prophylaxis is given.
Infection is almost always of the lungs, and rarely outside. The onset is subtle, at a peak incidence of 3-6 months of
age in HIV infected individuals, with a raised respiratory rate but no fever. There is a gradual increase in respiratory
distress and cyanosis with few clinical signs.
Chest x ray usually reveals marked changes of alveolitis, on some occasions looking like 'post-neonatal hyaline
membrane disease'.
Normal chest x ray is reported in 0-39% of cases in adults.
Diagnosis is most effectively by bronchi-alveolar lavage or brushings are even better by lung biopsy.
Treatment is high dose septrin and high dose steroids.
Copyright 2002 Dr Colin Melville